Behcet's Syndrome

Behçet's disease, also known as Silk Road disease, is a chronic (long-term) inflammatory condition, caused by vasculitis (blood vessel inflammation), which will affect and damage both your arteries and veins.

Causes of Behcet’s Syndrome

  • Scientists are still not exactly sure what causes this disease.

  • It may be an autoimmune disorder, meaning the body’s immune system mistakenly attacks healthy tissue with an inflammatory response.

  • Both HLA-B5 and HLA-B51 are gene markers that are sometimes present in patients with Behçet’s disease.

  • But there are some people who have this gene marker who don’t have Behçet’s disease.

  • Researchers are studying other genes related to immune function and think that infections (either bacteria or a virus) may play a role in triggering the disease in some people who have genetic markers that predispose them to Behçet’s.

Symptoms of Behcet’s Syndrome

  • Mouth sores, which occur at some time in all patients. They’re usually recurrent (keep coming back) and painful and affect almost all patients with Behçet’s disease. The sores look like the common canker sore, but are more numerous, frequent and painful. They’re often the first symptom that a person notices, and may occur long before any other symptoms appear. Mouth ulcers are seen on the lips, tongue and inside the cheek.

  • Genital sores look similar to mouth sores and may be painful. They aren’t as common as mouth sores. They appear on the scrotum (the sack enclosing the testes) in men and on the vulva (the external genital organs) in women.

  • Eye inflammation can cause pain, blurry vision, light sensitivity, tears or eye redness. Behçet’s disease may eventually lead to vision loss. Severe eye disease that can cause blindness is more common in the Middle East and Japan than in the United States.

  • Skin problems are a common symptom. They may look like acne or tender, coin-shaped nodules (erythema nodosum), or ulcers that may be shallow or deep and painful. A red bump or sore may develop if the skin is scratched or pricked. Doctors call this a positive pathergy test.

  • Joint pain is common. The ankles, knees, elbows and hips are most often affected. Joint inflammation causes swelling, redness and tenderness, though it usually doesn’t cause permanent damage.

  • Vein inflammation can lead to clots and blockages, or cause a vein to close completely. It can affect both superficial veins (those close to the surface of the skin) and deep veins. It may also affect the largest vein in the body (the vena cava), which could lead to serious health problems. These vein problems are caused by inflammation, not a flaw in the body’s clotting system.

Diagnosis of Behcet’s Syndrome

There is no single laboratory test that can diagnose Behçet’s disease. The diagnosis is usually made based on your symptoms, including how often (typically at least three times a year) oral ulcers come back. Plus, at least two more of the following:

  • Genital sores.

  • Eye inflammation.

  • Skin problems

  • A positive pathergy test (a pathergy test tests the immune system’s functionality; it’s performed by pricking the skin and then checking to see if a red bump develops a few days after the test).

Treatment of Behcet’s Syndrome

  • Corticosteroids, such as prednisone, are the main treatment. These drugs suppress inflammation and immune function.

  • Colchicine (Colcrys) can help with mouth sores, genital sores and possibly joint pain.

  • Other immunosuppressant drugs such as methotrexate (Trexall, Rasuvo), azathioprine (Imuran, Azasan), cyclophosphamide (Cytoxan, Neosar) and cyclosporine (Gengraf, Neoral, Sandimmune).

  • Also, biologics such as anti-TNF (Tumor Necrosis Factor), infliximab (Remicade), etanercept (Enbrel) and others, such as Tocilizumab (Actemra) are used in more severe cases.

  • Since these are immunosuppressant drugs, they can make you more susceptible to other infections.

  • Apremilast (Otezla), an oral medication (taken by mouth), is approved for treating mouth ulcers in patients with Behçet’s disease.

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